Transcranial magnetic stimulation in Behçet's disease: a cross-sectional and longitudinal study with 44 patients comparing clinical, neuroradiological, somatosensory and brain-stem auditory evoked potential findings.

OBJECTIVES: To compare neurological involvement in Behçet's disease as documented by transcranial magnetic stimulation (TMS) with clinical, neuroradiological, somatosensory (SEP) and auditory evoked potential (BAEP) findings. METHODS: Forty-four patients were studied over an 8 year period. Nine patients had follow-up studies done. TMS central motor conduction (CMC) studies to upper and lower limb muscles, brain magnetic resonance imaging (MRI), SEP, and BAEP testing were conducted. RESULTS: Thirty-nine patients had CMC slowing, decreased amplitude or absent motor evoked potentials (MEP); 5 of these patients were neurologically normal. Concordance of TMS results, clinical deficits, and MRI findings occurred in 36 of the 39 patients. SEP and BAEP testing proved non-complementary to MEP. Generally, follow-up studies revealed faster CMC and higher MEP amplitude. However, in two patients the CMC time to one target muscle became prolonged with diminished MEP amplitude over a period of 1.5-3 years. CONCLUSIONS: TMS can be useful in detecting and quantifying motor tract dysfunction in Behçet's disease and provides functional information complementary to imaging studies. TMS is more sensitive than either SEP or BAEP. Our longitudinal studies suggest that TMS studies may be valuable in monitoring disease activity or therapeutic response.

Craniocervical junction tuberculosis: a rare but dangerous disease.

BACKGROUND: Mycobacterium tuberculosis of the cervical spine is a rare but dangerous manifestation of extra-pulmonary tuberculosis. The clinical picture ranges from early, nonspecific, insidious symptoms to severe neurological complications and death, attributed to craniocervical junction instability and cervicomedullary compression. The different lines of management include antituberculous medication with traction and external fixation or adjunctive surgery (debridement and stabilization) in patients with severe or persistent neurological complications and/or vertebral instability. METHODS: We describe two patients with advanced craniocervical junction tuberculosis. The early clinical picture was nonspecific in Case 1 and obscured by psychiatric illness in Case 2. The detailed clinical and radiological findings, and the management, will be described. Involvement of the occipital condyles and foramen magnum, which has not been reported previously, will be demonstrated. RESULTS: Both cases underwent transoral biopsy, aspiration, and debridement of retropharyngeal abscess (granuloma). Histological and tissue culture studies proved the abscesses were tuberculous and anti-tuberculous medications were started. Case 1 showed complete resolution of the clinical and radiological findings. Case 2 developed cardiorespiratory arrest while in a halo jacket. He was resuscitated but remained quadriplegic and semiconscious; he developed nosocomial gram negative pneumonia. He was referred back to his local hospital where he died 1 year later. CONCLUSIONS: Tuberculosis is an infrequent but notable cause of cervicomedullary compression. It should be suspected in patients with infective spondylitis who are immunocompromised or reside in an area highly endemic for tuberculosis. Management strategies include antituberculosis medication, transoral biopsy and drainage of the abscess, traction and external fixation, posterior decompression, and internal fixation, according to the clinical and radiological findings.

Sildenafil (Viagra) A breakthrough in the management of an old problem or a risky drug.

Full text is available as a scanned copy of the original print version.

Sildenafi (Viagra).

Full text is available as a scanned copy of the original print version.

Biotin-responsive basal ganglia disease: a novel entity.

We describe a novel, biotin-responsive basal ganglia disease in 10 patients. At onset, it appears as a subacute encephalopathy, with confusion, dysarthria and dysphagia with occasional supranuclear facial nerve palsy or external ophthalmoplegia, and progresses to severe cogwheel rigidity, dystonia and quadriparesis. These symptoms disappear within a few days if biotin (5-10 mg/kg/day) is administered, and there are no neurological sequelae. They reappear within 1 month if biotin is discontinued. Patients diagnosed late, or who have had repeated episodes, suffer from residual symptoms such as paraparesis, mild mental retardation or dystonia. The numerous biochemical studies of intermediary metabolism, like the autoimmune and toxicological studies, enzyme assays including biotinidase, carboxylase and lysosomal activities, and bacterial and viral studies were all normal. The aetiology may be related to a defect in the transporter of biotin across the blood-brain barrier. The only consistent radiological abnormality was central necrosis of the head of the caudate bilaterally and complete, or partial, involvement of the putamen on brain MRI. This was present during the initial acute encephalopathy and remained unchanged during follow-up of 3-10 years. Although its aetiology is unknown, it is important to recognize this disease, since its symptoms may be reversed and the progression of its clinical course prevented simply by providing biotin.

Respiratory arrest: a complication of Arnold-Chiari malformation in adults.

We describe the cases of 2 adult patients who developed respiratory arrest and were found to have Arnold-Chiari malformation on magnetic resonance imaging. Following posterior fossa decompression both patients improved, though one of them subsequently died during sleep. Imaging of the craniocervical junction should be part of the workup of patients with unexplained apneic episodes.

Acute Wernicke's encephalopathy associated with hyperemesis gravidarum: magnetic resonance imaging findings.

A 25-year-old woman with hyperemesis gravidarum developed acute Wernicke's encephalopathy during prolonged intravenous fluid therapy without vitamin supplements. Delay in diagnosis led to a persistent severe neurological deficit, including coma. Gadolinium-diethylenetriaminepentaacetic acid-enhanced magnetic resonance imaging revealed symmetrical lesions around the aqueduct and fourth ventricle, which resolved after treatment with thiamine. She did not regain consciousness. This report demonstrates the diagnostic value of enhanced magnetic resonance imaging in acute Wernicke's encephalopathy.

Hemichorea in systemic lupus erythematosus: significance of MRI findings.

A young man with systemic lupus (SLE) developed hemichorea 13 years after the onset of his illness. For the first time in the course of his illness he had a positive test for anticardiolipin antibodies (aCL). Magnetic resonance imaging (MRI) of his brain showed lesions of presumed vascular cause in the ipsilateral basal ganglia. The findings support the contention that an immune phenomenon, invisible on proton imaging by MRI, is responsible for the striatal neuronal activation. Chorea, the clinical expression of this activation, was probably blocked on the side previously affected by vascular pathology.

Familial tremulous and myoclonic dystonia with white matter changes in brain magnetic resonance imaging.

We report two families with a disorder, probably autosomal recessive, characterized by tremor of juvenile onset, dystonia, and myoclonus with preserved cognitive, cerebellar, and peripheral nervous system functions. During 4 years' follow-up, mild spasticity appeared. Magnetic resonance imaging (MRI) revealed mild diffuse changes in the white matter. Central conduction times for visual, motor, and sensory systems were all prolonged. Extensive metabolic work-up failed to reveal lysosomal, peroxisomal, mitochondrial, or other metabolic abnormalities.

Evoked potential findings in Behçet's disease. Brain-stem auditory, visual, and somatosensory evoked potentials in 44 patients.

We studied 54 patients with Behçet's disease, 41 males and 13 females, mean age 28 years. Forty-four patients had auditory brain-stem evoked potential (BAEP) recordings, 39 had pattern reversal visual evoked potentials (VEP), 27 had median nerve somatosensory evoked potential (SEP) recordings, and 25 tibial nerve SEPs. BAEPs were abnormal in 16 patients (52%) with neurological manifestations and in 4 (31%) without, because of decreased amplitude of wave V, prolonged I-III or III-V interpeak latencies, or uncertain/absent waves III and/or V. Eleven patients (40%) with neurological symptoms and 3 patients (25%) without, had abnormal VEPs. Absent potentials, decreased amplitude, with or without prolonged P100 latency, were found in 75% of the cases, the rest had prolonged P100 latency only. Median SEPs were abnormal in 8 patients (38%) with neurological manifestations. Four patients (21%) had abnormal tibial SEPs. Decreased amplitude with or without mild slowing in central conduction was the predominant SEP abnormality. SEPs were normal in all patients without neurological symptoms. In total, 84% of patients with, and 38% of patients without, neurological symptoms had abnormalities of one or more EP modality. When used cautiously, EP studies in Behçet's disease might be helpful to separate neuro-Behçet from other disorders with similar symptomatology, to disclose subclinical CNS involvement, to evaluate and monitor CNS disease activity, and to provide objective measures of treatment response.

Myelopathy after intrathecal chemotherapy. A case report with unique magnetic resonance imaging changes.

BACKGROUND: Paraplegia caused by intrathecal chemotherapy has no known pathognomonic features and is a diagnosis of exclusion. METHODS: The authors reported the clinical and neuroimaging findings in one patient with this syndrome. RESULTS: The patient had severe paraplegia with urinary retention and impaired pain and touch sensation below T-10 with sparing of proprioception and vibration sense. Magnetic resonance imaging (MRI) scan showed diminished intensity throughout the central cervical spinal cord. Post-gadopentetate dimeglumine enhancement was scattered throughout the cervical spinal cord and in two areas of the dorsal spinal cord. Axial views of the cervical spinal cord showed that this enhancement was limited to the lateral columns. CONCLUSIONS: The MRI in myelopathy due to intrathecal chemotherapy may show a unique pattern of postgadopentetate dimeglumine enhancement limited to the lateral columns of the spinal cord. However, two recently encountered patients with the same syndrome did not show similar changes.

Subacute sclerosing panencephalitis. Imaging and clinical correlation.

Twenty-one patients at different clinical stages of subacute sclerosing panencephalitis were studied by magnetic resonance imaging and computed tomography. In patients in the early clinical stage, the imaging studies appeared normal. Within 6 months, diffuse or focal areas of high signal on T2-weighted magnetic resonance images were evident. Progressive hemispheric, cerebellar, and brainstem atrophy was seen later. Lesions of the deep nuclei were noted frequently in patients in the early and intermediate stages, with the lentiform involved more than the caudate. Magnetic resonance imaging was better than computed tomography in illustrating white matter and basal ganglia abnormalities. Radiographic progression occurred regardless of clinical course.

Fibrinolysis in pseudotumor cerebri.

Pseudotumor cerebri is a syndrome associated with diverse putative etiological factors that include chemicals such as vitamin A, tetracycline and estrogens or venous circulatory disturbances like sagittal or transverse sinus thrombosis. Diseases predisposing to thrombosis, such as polycythemia vera and essential thrombocythemia, were reported to cause sinus thrombosis and pseudotumor cerebri. This is a pilot study to investigate the possible role of hemostatic factors in the pathogenesis of pseudotumor cerebri. We studied nine patients with severe, recurrent, or refractory pseudotumor cerebri causing visual impairment and found abnormal euglobulin clot lysis time (prestress in all of them and post stress in seven). Digital subtraction angiography was suggestive of recanalized sinus thrombosis in only three patients. We conclude that abnormalities in the fibrinolytic system are present in a subset of patients with severe pseudotumor cerebri, which calls for further studies on venous circulatory pathogenesis of pseudotumor cerebri and the possible role of anticoagulants in such cases.

Magnetic resonance imaging of the brain in Wilson's disease.

Eight patients with Wilson's disease (WD) were studied by magnetic resonance imaging (MRI) of the brain; seven also underwent X-ray computed tomography (CT) of the brain. We describe the changes in the brain and try to correlate them with the clinical manifestations and progress of the disease. Six patients were symptomatic, with predominantly neurological problems. Two were asymptomatic, diagnosed upon screening siblings of index cases. Of the six symptomatic patients, five had basal ganglia lesions, combined in four with brain stem changes; in one with only brain stem abnormalities, clinical findings were minimal despite pronounced MRI changes. In three patients MRI abnormalities regressed following chelating therapy. MRI can contribute to documentation of early neurological involvement in WD, especially in patients with no abnormalities on CT. However, MRI changes may not correlate with clinical presentation or response to therapy.

Papilloedema in Behçet's disease: value of MRI in diagnosis of dural sinus thrombosis.

Behçet's disease is a multisystem disease characterised by the clinical triad of oral ulcers, genital ulcers and uveitis. Nervous system involvement is frequent and occasionally precedes other manifestations. Behcet's disease is not frequently considered in the differential diagnosis of papilloedema. We report four cases of Behcet's disease in which papilloedema occurred with or without dural sinus thrombosis. MRI is of great value in the investigation of such patients as it can demonstrate venous sinus thrombosis non-invasively or suggest the diagnosis by showing the associated parenchymal lesions secondary to small vessel pathology.

MRI findings in neuro-Behçet's disease.

We report MRI findings in 6 patients with Behçet's disease and CNS involvement. There were 3 different stages of imaging appearance: (1) During the acute illness, there were scattered areas of high signal intensity on T2-weighted images with predilection to the central structures of the cerebrum, the cerebral peduncles, and basis pontis. (2) During the recovery phase, most of these findings improved, but some white matter high signal areas persisted in the upper brainstem and peripheral subcortical white matter. Occasionally, findings were suggestive of microhematoma. (3) During the chronic phase, atrophy of posterior fossa structures became evident with decreased signal intensity suggestive of hemosiderin deposits.

Brain biopsy in tuberculoma: the risks and benefits.

In developing countries, 5 to 8% of the space-occupying lesions of the central nervous system are tuberculomas. Diagnosis can be difficult in the absence of extracranial tuberculosis; computed tomography is suggestive only. To assess the value of brain biopsies in tuberculomas, the records of 15 patients aged 6 to 80 years were reviewed. Histological confirmation was obtained in 15 patients, and acid-fast bacilli were cultured from 12 patients. Intracranial hypertension was the principal sign in 11 patients; other neurological signs were related to the location of the tuberculoma. One patient had evidence of extracranial tuberculosis. Biopsy-related complications consisted of an epidural hematoma in 1 patient and hydrocephalus in another; both required additional surgery. One case of tuberculous meningitis was probably related to surgery and poor drug compliance. There was no postoperative mortality. Thirteen patients (2 were lost to follow-up) were cured after an average of 16 months of antituberculous therapy. It was concluded that the brain biopsy is useful in diagnosing tuberculoma but that there is some associated risk.